Diagnosis and treatment of primary biliary cholangitis

Abstract

<jats:p> Primary biliary cholangitis is a cholestatic, chronic autoimmune liver disease with a wide individual variation in disease progression. The diagnosis is predominantly based on chronic elevation of alkaline phosphatase and the presence of anti-mitochondrial antibodies or other specific antinuclear antibodies (i.e. anti-gp210 and anti-sp100). Even in early-stage disease, health-related quality of life can be severely impaired by symptoms such as pruritus, fatigue, and sicca syndrome and metabolic bone disease should be assessed and treated. The prognosis of the disease is, however, largely determined by the development of cirrhosis and its complications. Ursodeoxycholic acid is associated with an improved prognosis and should be initiated and continued in all patients. Clinical outcome is related to the biochemical response to ursodeoxycholic acid, but the prognosis of those with an incomplete response is still better than those who remain untreated. Obeticholic acid was recently approved as second-line treatment and bezafibrate may serve as an adequate off-label alternative, particularly in patients with pruritus. Preliminary data suggest an additive effect of triple therapy with ursodeoxycholic acid, obeticholic acid, and bezafibrate, whereas other promising drugs are being evaluated in clinical trials. </jats:p>

In Silico Prediction and Pharmacokinetic Comparison of Ursodeoxycholic Acid and Obeticholic Acid in the Management of Primary Biliary Cholangitis

“…30% of western patients and 32%-44.7% of Chinese patients failed to give an adequate response to UDCA therapy. 21 Ageing is a patient-specific factor that is not largely considered but can also give rise to unintended interactions due to changes in systemic absorption with advancing age. A comparative study of P.K.…”

Factors Explaining the Coincidence of Smoldering Multiple Myeloma and Primary Biliary Cholangitis: A Case Report

“…Primary biliary cholangitis (PBC), however, is a rare autoimmune disorder with an estimated 12-year prevalence in the United States of 29.3 per 100,000 individuals [ 4 ]. A patient is diagnosed with PBC based on the clinical presentation, detectable anti-mitochondrial antibody (AMA) levels, abnormal liver function test (LFT) parameters for six months [ 5 , 6 ], and histological findings showing chronic nonsuppurative inflammation of interlobular and septal bile ducts [ 5 ].…”

Factors associated with severity and persistence of fatigue in patients with primary biliary cholangitis: study protocol of a prospective cohort study with a mixed-methods approach (SOMA.LIV)

“…Elevated markers of cholestasis, presence of circulating anti-mitochondrial antibodies or other specific antinuclear autoantibodies, and in selected cases, typical liver histology is used for diagnosis. 7 The course of PBC is most often accompanied by the occurrence of non-specific symptoms such as fatigue, sicca or pruritus with up to 70% of patients reporting such symptoms. 8 The pathogenesis of PBC is incompletely understood, but genetic predisposition combined with external triggers such as environmental exposure, bacterial metabolism of xenobiotics or gut microbiota probably initiates the disease.…”